Sepúlveda, MaríaArmangué, ThaísSola Valls, NuriaArrambide, GeorginaMeca Lallana, José E.Oreja-Guevara, CeliaMendibe, MarAlvarez de Arcaya, AmayaAladro, YolandaCasanova, BonaventuraOlascoaga, JavierJiménez Huete, AdolfoFernandez Fournier, MireyaRamió Torrentà, LluísCobo Calvo, ÁlvaroViñals, MontserratAndrés, Clara deMeca Lallana, VirginiaCervelló, AngelesCalles, CarmenBarón Rubio, ManuelRamo Tello, CristinaCaminero, AnaMunteis, ElviraAntigüedad, Alfredo R.Blanco, YolandaVilloslada, PabloMontalbán Gairín, XavierGraus Ribas, FrancescSaiz Hinarejos, AlbertSpanish NMO Study Group2018-12-102018-12-102016-06https://hdl.handle.net/2445/126844Objective: To (1) determine the value of the recently proposed criteria of neuromyelitis optica (NMO) spectrum disorder (NMOSD) that unify patients with NMO and those with limited forms (NMO/LF) with aquaporin-4 immunoglobulin G (AQP4-IgG) antibodies; and (2) investigate the clinical significance of the serologic status in patients with NMO. Methods: This was a retrospective, multicenter study of 181 patients fulfilling the 2006 NMO criteria (n = 127) or NMO/LF criteria with AQP4-IgG (n = 54). AQP4-IgG and myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) antibodies were tested using cell-based assays. Results: Patients were mainly white (86%) and female (ratio 6.5:1) with median age at onset 39 years (range 10-77). Compared to patients with NMO and AQP4-IgG (n = 94), those with NMO/LF presentedmore often with longitudinally extensive transverse myelitis (LETM) (p<0.001), and had lower relapse rates (p = 0.015), but similar disability outcomes. Nonwhite ethnicity and optic neuritis presentation doubled the risk for developing NMO compared with white race (p = 0.008) or LETM presentation (p = 0.008). Nonwhite race (hazard ratio [HR] 4.3, 95% confidence interval [CI] 1.4-13.6) and older age at onset were associated with worse outcome (for every 10-year increase, HR 1.7, 95% CI 1.3-2.2). Patients with NMO and MOG-IgG (n = 9) had lower female: male ratio (0.8:1) and better disability outcome than AQP4-IgG-seropositive or double-seronegative patients (p<0.001). Conclusions: In patients with AQP4-IgG, the similar outcomes regardless of the clinical phenotype support the unified term NMOSD; nonwhite ethnicity and older age at onset are associated with worse outcome. Double-seronegative and AQP4-IgG-seropositive NMO have a similar clinical outcome. The better prognosis of patients with MOG-IgG and NMO suggests that phenotypic and serologic classification is useful.9 p.application/pdfengcc by-nc-nd (c) American Academy of Neurology, 2016http://creativecommons.org/licenses/by-nc-nd/3.0/es/Malalties del nervi òpticMalalties del sistema nerviós centralOptic nerve diseasesCentral nervous system diseasesinfo:eu-repo/semantics/article2018-07-25info:eu-repo/semantics/openAccess